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Flashcards in Vascular tumors Deck (17)
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1

What syndrome is associated with facial hemangioma?

PHACES

2

What anomalies are associated with PHACES syndrome (facial segmental IH)?

P- posterior fossa defects
H- hemangioma (facial segmental)
A - arterial anomalies (intracranial)
C- Cardiac anomalies (aorta)
E- eye anomalies
S- sterna clefts

3

What potentially life threatening anomaly is found with hemangioma small of the mandible?

Airway hemangioma- biphasic stridor, usually less than 6 months of age

4

Most common vascular tumors?

Infantile hemagioma
Rapidly involuting congenital hemangioma
Non involuting congenital hemangioma
Kaposiform hemangioendothelioma
Tufted angioma (angioblastoma)

5

How do you subtype infantile hemangiomas?

Distribution- Focal, multifocal, segmental
Depth- superficial, mixed, deep (below dermis)

6

Phases of IH?

Proliferation 0-12 months
Involution 1-10 yrs
Involuted

7

Pelvis Syndrome?

Perineal hemangioma
External genital malformation
Lipomyelomeningocele
Vascular anomalies
Imperforate anus
Skin tag

8

Sacral syndrome

Spinal dysraphism
Anogenital disorders
Cutaneous disorders
Renal
Associated lumbosacral hemangioma

9

Lumbar Syndrome

Lower body hemangioma
Urogenital
Myelopathy
Bony deformities
Anorectal
Arterial
Renal

10

Treatment for ulceration with IH?

Surgery
Topical Abx, hydrocoloid dressing
Steroids

Laser ablation

Propranolol

11

When do you screen for hepatic hemangioma?

- Large hemangioma in the H&N
- Lumbosacral hemangioma
- > 5 cutaneous hamngioma
- severe thrombocytopenia

12

What are the subtypes of hepatic hemangioma?

Solitary - rapidly involuting congenital hemangioma (glut-1 negative)

Mutlifocal- IH (glut-1 +) - associated cutaneous IH and heart failure

Diffuse - IH (glut-1 +) - associated hypothyroid and mortality

13

IH Theraputic options?

Observation
Drugs
Laser beams
Surgery

Drugs- propranolol, vincrisinte, INF-a

14

Congenital hemangioma types?

Rapidly involuting (usually resolved by 12-18 months)
Non-involuting (does not change with time)- requires surgery

15

Features and treatment of Kaposiform hemgioendothelioma & tufted angioma

KH - present at birth - expanding red plaque on trunk or in the mediastinum/retroperitonum

TA- present at birth- trunk/neck, dusky red plaque, may resolve or expand.

-both are associated with Kasabach-Merritt

-treatment- steroids, sirilimus, vincrisitne

16

Angiosarcoma prognostic factors?

Deep/visceral location
>5 cm
Positive margins
Local recurrence
Mets

17

Angiosarcoma treatment?

Resection to negative margins
-may require neoadjuvent or adjuvant chemo